Background The current study was the first to investigate the association between burn-out and Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL),...Show moreBackground The current study was the first to investigate the association between burn-out and Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), a rare hereditary microangiopathy caused by mutations in the NOTCH3-gene. Previous research investigating burn-out and related factors provided evidence for a possible association between burn-out and mild cognitive impairment (MCI), lacune count, disease severity, stroke incidence and level of education. The current study investigated whether burn-out is an early symptom of incipient CADASIL. Methods The study population consisted of 151 individuals aged 20 years and older, with cysteine altering NOTCH3-mutations. For the current study, an MRI-scan of the brain and neuropsychological assessment (attention, executive functioning and memory) were performed. Results A history of burn-out was found in 33 participants. History of burn-out was associated with both MCI (p = .025, OR [Odds Ratio] = 3.46) and disease severity (p < .010, OR = 2.854), but not with lacune count (p = .926, OR = 1.038), stroke (p = .823, OR = .897) or level of education (p = .725, OR = 1.163). Conclusion In this sample of persons with a NOTCH3-mutation, burn-out was associated with MCI, and with disease severity. Hypothetically, in persons with a NOTCH3-mutation, burnout could be a consequence of MCI, and thus an early symptom of CADASIL. The ability to recognise the clinical onset of CADASIL more timely could be helpful in the clinical setting, because this could allow for better guidance and education of the patient and their support system.Show less